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- Title
Primary brain amyloidoma, both a neoplastic and a neurodegenerative disease: a case report.
- Authors
Löhr, Mario; Kessler, Almuth F.; Monoranu, Camelia-Maria; Grosche, Jens; Linsenmann, Thomas; Ernestus, Ralf-Ingo; Härtig, Wolfgang
- Abstract
<bold>Background: </bold>Scattered extracellular deposits of amyloid within the brain parenchyma can be found in a heterogeneous group of diseases. Its condensed accumulation in the white matter without evidence for systemic amyloidosis is known as primary brain amyloidoma (PBA). Although originally considered as a tumor-like lesion by its space-occupying effect, this condition displays also common hallmarks of a neurodegenerative disorder.<bold>Case Presentation: </bold>A 50-year-old woman presented with a mild cognitive decline and seizures with a right temporal, irregular and contrast-enhancing mass on magnetic resonance imaging. Suspecting a high-grade glioma, the firm tumor was subtotally resected. Neuropathological examination showed no glioma, but distinct features of a neurodegenerative disorder. The lesion was composed of amyloid AL λ aggregating within the brain parenchyma as well as the adjacent vessels, partially obstructing the vascular lumina. Immunostaining confirmed a distinct perivascular inflammatory reaction. After removal of the PBA, mnestic impairments improved considerably, the clinical course and MRI-results are stable in the 8-year follow-up.<bold>Conclusion: </bold>Based on our histopathological findings, we propose to regard the clinicopathological entity of PBA as an overlap between a neoplastic and neurodegenerative disorder. Since the lesions are locally restricted, they might be amenable to surgery with the prospect of a definite cure.
- Subjects
NEURODEGENERATION; REPORTING of diseases; BRAIN; MAGNETIC resonance imaging
- Publication
BMC Neurology, 2019, Vol 19, Issue 1, pN.PAG
- ISSN
1471-2377
- Publication type
journal article
- DOI
10.1186/s12883-019-1274-x